A 65-year-old woman presented to her general practitioner with a 2-month history of mild abdominal pain, change in bowel habits and unintentional weight loss. She reported no nausea, fever, night sweats or fatigue. Her past medical history was unremarkable, except for past hepatitis B virus infection. She was not taking any medication at that time.
Abdominal examination revealed mild tenderness, without signs of peritonitis. Laboratory studies were significant for mild microcytic anemia and elevated erythrocyte sedimentation rate. Computed tomography (CT) scan of the abdomen revealed the presence of a 13-cm duodenojejunal mass infiltrating adjacent organs (Figure 1a). An endoscopic biopsy was made. On microscopic examination, the mass was composed of monomorphic small lymphocytes positive for CD3, CD8 and CD56 consistent with a diagnosis of MEITL (Figure 2). Staging positron emission tomography (PET) scan revealed increased uptake in multiple abdominal lymph nodes, hepatic flexure, ascending colon and spleen. Bone marrow biopsy showed no signs of disease.
Surgical resection was not performed, due to the aortic wall infiltration. The patient received an anthracycline-containing regimen, inspired to the SNLG protocol [3], achieving partial remission, allowing surgical resection of the residual mass by laparotomy. She was then considered for autologous stem cell transplant (ASCT) consolidation, which was halted due to the development of severe renal insufficiency, with features of acute tubular necrosis and interstitial nephritis, poorly responsive to high-dose corticosteroids.
Thirteen months later CT- and PET-scans revealed recurrence of lymphoma, with multiple lesions, located in the bowel and retroperitoneum (Figure 1b). Relapse was accompanied by renal and cutaneous manifestations (i.e., kidney failure and palpable purpura) suggestive of paraneoplastic vasculitis, with sudden deterioration in patient general conditions. The patient received a first dose of 1500U/m2 (2750U total dose) single-agent PEG-asparaginase as salvage therapy. Skin manifestation completely resolved and renal function recovered. Ultra-sound of the abdomen showed significant shrinkage of the biggest lesions, consistent with partial response. However, the patient developed a grade 4 hepatotoxicity, causing treatment delay until resolution to grade 1. A reduced flat dose of 1500U PEG-asparaginase was then resumed. Unfortunately, the subsequent cycle was complicated by a thromboembolic event and she died of massive pulmonary embolism, 3 months after the start of PEG-asparaginase therapy.